Amegakaryocytic Thrombocytopenia With Duplication of Part of the Long Arm of Chromosome 3

Hallett, Janet; Martell, R. W.; Sher, Clive; Jacobs, Peter

doi:10.1111/j.1365-2141.1989.tb04269.x

Cited by 12 publications

(3 citation statements)

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“…On rare occasions a duplication of chromosome 3 and presence of Ph 1 (Philadelphia chromosome, e.g. t(9;22)) without chronic myeloid leukemia were described (11, 12). We confirmed the presence of BCR‐ABL rearrangement lacking phenotypic expression on three occasions in a 3‐yr period.…”

Section: Discussionmentioning

confidence: 99%

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Acquired amegakaryocytic thrombocytopenia associated with proliferation of γ/δ TCR⁺ T‐lymphocytes and a BCR‐ABL (p210) fusion transcript

Čolović

Pavlović

Kraguljac

et al. 2004

European J of Haematology

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Acquired amegakaryocytic thrombocytopenia (AATP) in adults is a rare disorder characterized by severe thrombocytopenia and decreased or absent megakaryocytes in an otherwise normal bone marrow. We present a 44-yr-old man in whom the diagnosis of AATP was established in January 2001. Immunophenotyping of the peripheral blood lymphocytes showed a relative increase in the subpopulation of gamma/delta T-cell receptor (TCR) positive (gamma/delta TCR(+)) and (CD4, CD8) negative T lymphocytes, and PCR suggested a monoclonal pattern of TCR gamma chain gene rearrangement. Cytogenetic examination of his bone marrow cells showed a normal male karyotype but RT-PCR analysis revealed a BCR-ABL (p210) fusion transcript. The inhibition of CFU-Mk growth mediated by the patient's T lymphocytes indicated that the pathogenic mechanism for AATP could be an immunological attack on megakaryocyte progenitors where the gamma/delta TCR-positive T lymphocytes are directly involved. The case emphasizes the complex association of T-lymphocyte monoclonal proliferation and AATP.

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Section: Discussionmentioning

confidence: 99%

“…It can also be an early manifestation of the failure of hematopoietic stem cell pool, as it was found that some patients are evolving to aplastic anemia or acute leukemia. In the literature 31 cases of AAT, more frequently in females, were reported (1–5, 8–12).…”

Section: Discussionmentioning

confidence: 99%

Acquired amegakaryocytic thrombocytopenia associated with proliferation of γ/δ TCR⁺ T‐lymphocytes and a BCR‐ABL (p210) fusion transcript

Čolović

Pavlović

Kraguljac

et al. 2004

European J of Haematology

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“…The human Evi-1 gene has been mapped to chromosome 3q26 [32], and its abnormal expression is related to structural abnormalities of the chromosome region 3q21 to 3q26 [33]. Since elevated thrombopoiesis is observed among patients with these abnormalities [34,35], Evi-1 was thought to be a regulator of hematopoietic factor(s) for platelet production. Cotransfection experiments with Evi-1 showed that there were significant effects on the human TPO promoter activity.…”

Section: Characterization Of the Consensus Sequences Recognized By Evmentioning

confidence: 99%

Gene Expression and Transcriptional Regulation of Thrombopoietin

Ogami

1996

Stem Cells

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Thrombopoietin (TPO) is predominantly expressed in the liver among various tissues that express TPO transcripts. To investigate the transcriptional regulation of the human TPO gene in the liver, we determined the major transcription initiation site by means of 5'-RACE and Northern blotting. From these analyses, we concluded that TPO gene transcription started at various points, and the transcription initiation sites of the human TPO gene were localized downstream, close to a point we determined by S1 nuclease mapping. The human TPO promoter region contains consensus sequences of GATA, Evi-1, and Ets binding sites. We used the hepatocellular carcinoma cell line, HepG2, that expresses TPO mRNA to analyze its promoter activity by transfecting various reporter plasmids containing a sequentially 5'-deleted human TPO promoter. Although GATA binding factors increased the promoter activity, their effect was independent of the GATA binding consensus sequence. On the other hand, Evi-1 did not affect transcription. Moreover, we defined the core promoter region, in which an Ets binding consensus sequence was located. The deletion or mutation of the Ets binding site resulted in a loss of the promoter activity. These results suggested that TPO is regulated by the Ets family of transcription factors.

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Thrombozytenbildungs- und Verteilungsstörungen: Thrombozytosen und Thrombozytopenien

Heimpel¹,

Seifried²

1999

Hämostaseologie

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Amegakaryocytic Thrombocytopenia With Duplication of Part of the Long Arm of Chromosome 3

Cited by 12 publications

References 11 publications

Acquired amegakaryocytic thrombocytopenia associated with proliferation of γ/δ TCR⁺ T‐lymphocytes and a BCR‐ABL (p210) fusion transcript

Acquired amegakaryocytic thrombocytopenia associated with proliferation of γ/δ TCR⁺ T‐lymphocytes and a BCR‐ABL (p210) fusion transcript

Gene Expression and Transcriptional Regulation of Thrombopoietin

Thrombozytenbildungs- und Verteilungsstörungen: Thrombozytosen und Thrombozytopenien

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Amegakaryocytic Thrombocytopenia With Duplication of Part of the Long Arm of Chromosome 3

Cited by 12 publications

References 11 publications

Acquired amegakaryocytic thrombocytopenia associated with proliferation of γ/δ TCR+ T‐lymphocytes and a BCR‐ABL (p210) fusion transcript

Acquired amegakaryocytic thrombocytopenia associated with proliferation of γ/δ TCR+ T‐lymphocytes and a BCR‐ABL (p210) fusion transcript

Gene Expression and Transcriptional Regulation of Thrombopoietin

Thrombozytenbildungs- und Verteilungsstörungen: Thrombozytosen und Thrombozytopenien

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Acquired amegakaryocytic thrombocytopenia associated with proliferation of γ/δ TCR⁺ T‐lymphocytes and a BCR‐ABL (p210) fusion transcript

Acquired amegakaryocytic thrombocytopenia associated with proliferation of γ/δ TCR⁺ T‐lymphocytes and a BCR‐ABL (p210) fusion transcript